Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Possible effects and side-effects of 'factor VIII inhibitor bypassing activity (FEIBA)' were tested on 7 haemophiliacs with high-titre antibody to factor VIII (resistant haemophilia). FEIBA was administered both to hospitalised patients and as part of a home therapy programme. Serious side-effects, attributable to FEIBA, were one episode of hypersensitivity and, possibly, hepatitis. Our preliminary data suggest that early injection of FEIBA, using considerably lower doses than suggested by the manufacturer, may shorten the duration of immobilisation of haemophiliacs with inhibitor against factor VIII in comparison with infusion of factor VIII and/or supportive care alone, at least following musculoskeletal bleeding. A decrease or complete disappearance of the inhibitor was observed in all patients receiving FEIBA alone.
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PMID:Effects of low-dose 'factor VIII inhibitor bypassing activity (FEIBA)' in resistant haemophilia. 677 72

Twenty-four children and adolescents who have been receiving home treatment for haemophilia A and B, and were followed up for a median period of five years, have been assessed for physical activity, social adjustment, range of joint movement and infection with hepatitis viruses. They were treated with cryoprecipitate from 1972 to 1977, and since then with factor-VIII concentrates. The average dose of factor VIII was 20 units/kg body mass. It was found that there was near normal range of physical activity and school performance, and, in virtually all families, near normal family function could be preserved. Approximately one-third of the patients showed impairment of the normal range of joint movement in flexion and extension. Although there was no clinical evidence of liver disease, elevated aspartate aminotransferase (AST) levels were found in 14 patients. Evidence of past, or present, infection with hepatitis B was found in 19 patients, and of infection with hepatitis A in seven patients. Home treatment is associated with a reduced level of disability from haemophilia, but transfusion therapy continues to be associated with a high rate of liver function abnormalities, probably of infectious origin.
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PMID:Home treatment of haemophilia. A follow-up study. 679 76

In 144 patients with bleeding disorders and in 11 patients with normal coagulation, 517 wound sealings were performed using the fibrin adhesion system (FAS) with the primary object of local hemostasis. Excellent results were obtained in patients with bleeding disorders caused by impaired thrombocyte function or anticoagulant therapy. Replacement therapy or the withdrawal of anticoagulant medication was thereby avoided. Ultimate hemostasis in patients with hemophilia was achieved by fibrin sealing in combination with low-dose replacement therapy with clotting factor concentrates. Though the risk of hepatitis transmission cannot be completely ruled out, the advantages, such as excellent tissue tolerance, complete resorption, and a wide spectrum of practical uses, speak in favor of the use of this physiologic tissue adhesive.
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PMID:The use of the fibrin adhesion system for local hemostasis in oral surgery. 698 Sep 78

Chronic liver disease has become a significant complication of the therapy of hemophilia disorders. We describe two patients with hemophilia A and hepatitis B virus hepatitis who progressed to cirrhosis with bleeding esophageal varices. Each underwent distal splenorenal shunt under plasma concentrate therapy without difficulty. One patient died 19 months after operation and unsuspected hepatocellular carcinoma was found at autopsy. These cases illustrate the potential severity of liver disease in hemophilia and the ability to safely perform surgery for portal hypertension if required.
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PMID:Cirrhosis, variceal bleeding, and distal splenorenal shunt in hemophilia A. 712 25

The Singapore Blood Transfusion Service (BTS) collects, processes and distributes blood and blood components to all hospitals. It also provides clinical and diagnostic haematological services to the hospitals. Blood donation is based on an entirely voluntary system. Blood donor incentives include a scheme of medical benefits for regular blood donors. Major advances in the 1960s include the introduction of disposable plastic blood packs for blood collections which permitted blood components to be prepared in a closed system. The development of a mobile blood collection service has enabled many more donor sources to be tapped. Hepatitis B Surface Antigen (HBsAg) screening of blood donors was introduced in 1973 as local studies showed that HBsAg positive blood was associated with a high risk of hepatitis. HBsAg positive donors are permanently excluded from the donor panel. BTS provides reference services for Immunohaematology, Coagulation problems, a clinical and diagnostic haematology service and a haemophilia centre. A computerized system of blood donor call up and the introduction of automation in the transfusion and haematology laboratories have helped BTS cope with an increasing volume of work and at the same time restrain manpower increases.
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PMID:The development of blood services in Singapore. 713 22

Liver biopsies were performed in 5 boys aged between 2 and 9 years with severe classical haemophilia who had persistently abnormal liver function tests. Abnormal histology was present in all; 4 had chronic persistent hepatitis and the fifth chronic aggressive hepatitis with early cirrhosis. Evidence of previous hepatitis B infection was present in one patient, 3 had antibodies to hepatitis, A, and 2 had subnormal levels of alpha-1-antitrypsin. Haemobilia occurred as a late complication of biopsy in one. The significance of these findings in young boys is discussed, as is the role of exposure to factor VIII containing blood products. It is concluded that cryoprecipitate should be used in preference to large pool factor VIII concentrates in children with haemophilia.
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PMID:Liver disease complicating severe haemophilia in childhood. 743 4

Inherited coagulation protein deficiencies associated with bleeding diatheses may present with spontaneous bleeding early in life, or may not be recognized until the development of hemorrhage after trauma or surgery. Diagnostic evaluation with coagulation screening tests, followed by confirmation with coagulation factor assays, is essential for appropriate management. For moderate-to-severe hemophilia, treatment includes coagulation factor replacement with purified, plasma-derived coagulation factor, or in the case of hemophilia A, factor VIII concentrate produced with recombinant techniques. Increased use of pharmacologic agents such as desmopressin acetate for patients with mild hemophilia A or type 1 von Willebrand's disease has allowed physicians to treat patients without the risk of infectious complications from plasma-derived factor concentrates. In addition to the management of the inherited bleeding disorders, patients may also require management of human immunodeficiency virus infection, hepatitis, and coagulation factor inhibitors. Issues for the coming years will include continued work to ensure product safety, the role of prophylactic treatment to prevent longterm disabilities, and the application of gene therapy to the management of bleeding disorders.
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PMID:Treatment of inherited coagulation disorders. 887 17

Non-A non-B (NANB) hepatitis plays a major role in liver disease in hemophiliacs. HCV is known to be the predominant cause for blood-borne NANB hepatitis. A cross-sectional study for anti-HCV and anti-HIV-1 antibodies in sera, presence of HBsAg in sera and liver function tests was conducted in 116 male patients with hemophilia (mean age: 14.6 years) in order to study the impact of hepatitis C as well as the significance of concurrent hepatitis B and HIV infection on the liver disease in hemophilic children and adolescents. 56.9% of the patients tested seropositive for anti-HCV; the mean age of the anti-HCV positive group was higher than that of the anti-HCV seronegative group (15.9 versus 11.9 years). Seropositivity to anti-HCV was more often associated with abnormal liver function than it was found in the seronegative group (37.9% versus 17%). Eight of nine patients positive for anti-HCV and HBsAg showed abnormal liver function tests. 68.9% of the anti-HIV-1 positive patients were also anti-HCV positive compared to 44.8% of the anti-HIV-1 negative patients. The liver function tests revealed an abnormal result in 47% of the anti-HIV-1 positive patients compared to 20.7% in the anti-HIV-1 negative group. In conclusion, a high seroprevalence for anti-HCV is detected in young patients with hemophilia which is associated with liver disease in a considerable number of patients when assessed by liver function tests. The coinfection of HCV and HBV seems to increase the risk of liver as also does concurrent HIV-1 infection, which is assumed to contribute to liver disease in a yet unexplained way.
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PMID:Hepatitis C contributes to liver disease in children and adolescents with hemophilia. 751 69

Hemophilia A and B patients seen at nine US regional treatment centers were tested for serologic markers of hepatitis B virus (HBV), hepatitis C virus (HCV), and hepatitis delta virus (HDV) during 1987 and 1988. Because human immunodeficiency virus (HIV) infection, a potentially confounding variable, was present in 53% of the group, the population was divided by HIV status for analysis purposes. In the HIV-positive group (N = 382), less than 1% had not been infected with HBV, HCV, or HDV, whereas 75% had evidence of infection with HBV and 98% with HCV. HBsAg, a marker of active HBV infection, was present in 12% of subjects; 96% of these were HCV positive. Anti-HDV was detected in 35 subjects (9.1%); all were anti-HBc positive. Ten of the 35 (29%) also were positive for IgM anti-HDV, indicating current infection. All 10 were HBsAg positive and 7 of the 9 tested were HDV RNA positive. Severe/moderate hemophilia B patients were more likely to have experienced an HBV infection and to be anti-HDV positive than were similar hemophilia A patients (22% v 8%, P < .05). In the HIV-negative group (N = 345), the subjects were younger and had less severe hemophilia than the HIV-positive patients. No evidence of HBV, HCV, or HDV infection was found in 18%, whereas 33% had experienced HBV infection and 79% were anti-HCV positive. Within this group, 4% were HBsAg positive. All 13 subjects with anti-HDV (4% of the HIV-negative group) also possessed anti-HBc. One (7.7%) was IgM anti-HDV positive and the serum from another contained HDV RNA. Both of these individuals were HBsAg positive. As in the HIV-positive group, severe/moderate hemophilia B patients were more likely to be HBV and HDV positive than were hemophilia A patients (9% v 3%, P < .05). A prevalence study of viral hepatitis in a large US hemophilic population showed that active infection with HCV is common, occurring in 89% of all study patients regardless of HIV status. Evidence of active HBV infection was found in 8%; 19% of these were actively infected with HDV. HDV was more common in hemophilia B patients after controlling for disease severity.
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PMID:A multicenter study of viral hepatitis in a United States hemophilic population. 767 17

Hemophilia A is caused by a deficiency of factor-VIII procoagulant (fVIII) activity. The current treatment by frequent infusions of plasma-derived fVIII concentrates is very effective but has the risk of transmittance of blood-borne viruses (human immunodeficiency virus [HIV], hepatitis viruses). Use of recombinant DNA-derived fVIII as well as gene therapy could make hemophilia treatment independent of blood-derived products. So far, the problematic production of the fVIII protein and the low titers of the fVIII retrovirus stocks have prevented preclinical trials of gene therapy for hemophilia A in large-animal models. We have initiated a study of the mechanisms that oppose efficient fVIII synthesis. We have established that fVIII cDNA contains sequences that dominantly inhibit its own expression from retroviral as well as from plasmid vectors. The inhibition is not caused by instability of the fVIII mRNA (t1/2, > or = 6 hours) but rather to repression at the level of transcription. A 305-bp fragment is identified that is involved in but not sufficient for repression. This fragment does not overlap the region recently identified by Lynch et al (Hum Gene Ther 4:259, 1993) as a dominant inhibitor of RNA accumulation. The repression is mediated by a cellular factor (or factors) and is independent of the orientation of the element in the transcription unit, giving the repressor element the hallmarks of a transcriptional silencer.
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PMID:Expression of the blood-clotting factor-VIII cDNA is repressed by a transcriptional silencer located in its coding region. 772 75


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